History of the infantile hepatic hemangioma: From imaging to generating a differential diagnosis

Abstract

We aim to provide an up-to-date summary of infantile hepatic hemangioma (IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver. Eligible peer-reviewed articles on hepatic infantile hemangiomas, published between 2000 and 2015, were reviewed for this study. IHH is the most common hepatic vascular tumor in children. Once a liver mass is identified in an infant, the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma, hepatoblastoma, metastatic neuroblastoma, so careful physical examination, imaging studies, and, if indicated, tumor markers and biopsy, are of pivotal importance to ascertain the correct diagnosis. Despite the benign nature of IHHs, some of these lesions may demand medical and/or surgical intervention, especially for multiple and diffuse IHH. Complications can include hepatomegaly, hypothyroidism and cardiac failure. Therefore, a close follow-up is required until complete involution of the lesions. We propose an algorithm to guide the physicians towards the proper management of hepatic lesions.

Keywords: Children; Hepatic hemangiomas; Infant; Vascular tumors.

Figure 1

Focal infantile hepatic hemangiomas. A: Coronal T2 weighted MRI image through the abdomen of an 8-wk-old boy revealing a large hyperintense mass arising from the liver (arrow); B: Abdominal USG of the same patient at 17-mo-old shows a minimal residual scar (demarcated by calipers). USG: Ultrasonography; MRI: Magnetic resonance imaging.

Figure 2

Multiple infantile hepatic hemangiomas. Coronal T2 weighted MRI image through the upper abdomen in a 5-mo-old girl depicts multiple well-defined, T2 hyperintense masses in the liver (arrows). This was consistent with multifocal infantile hepatic hemangiomas. MRI: Magnetic resonance imaging.

Figure 3

Diffuse infantile hepatic hemangiomas. T2 weighted axial MRI image of a 7-d-old with diffuse hemangiomas. Note the innumerable T2 hyperintense masses throughout the liver with central hypo-intense central regions (arrow). MRI: Magnetic resonance imaging.

Figure 4

A schematic algorithm to guide physicians through correct diagnosis and management of hepatic lesions. IHH: Infantile hepatic hemangioma; CT: Computed tomography; MRI: Magnetic resonance imaging; DDx: Differential diagnosis; GLUT-1: Glucose transporter-1; AFP: α-fetoprotein; KHE: Kaposiform Hemangioendothelioma.

Figure 5

Hepatoblastoma. Axial computed tomography scan after the injection of IV contrast material in a 7-mo-old girl demonstrates a poorly enhancing large mass within the liver (arrow).

Figure 6

Mesenchymal hamartoma. 15-mo-old with abdominal distention: Axial computed tomography scan after the administration of IV contrast material demonstrates a large multi-cystic mass arising from the liver (arrow). Additional mixed solid and cystic elements are present laterally in the expanded left hepatic lobe.